What are the treatment options for managing Amyotrophic Lateral Sclerosis (ALS) symptoms?

Management of ALS Symptoms

ALS symptom management requires immediate multidisciplinary care from diagnosis, with riluzole as the only FDA-approved disease-modifying therapy, combined with aggressive nutritional support, respiratory interventions, and palliative care to maximize survival and quality of life. 1, 2

Disease-Modifying Pharmacotherapy

• Riluzole 50 mg twice daily (taken at least 1 hour before or 2 hours after meals) is the primary disease-modifying treatment for ALS, modestly extending survival 1, 3
• Monitor serum aminotransferases before and during treatment; discontinue if liver dysfunction develops or if baseline elevations exceed 5 times upper limit of normal 1
• Edaravone represents a second approved disease-modifying agent that slightly increases life expectancy 4
• Common adverse reactions include asthenia, nausea, dizziness, decreased lung function, and abdominal pain (≥5% incidence) 1

Respiratory Management

Initiate non-invasive ventilation (NIV) when forced vital capacity (FVC) falls below 80% of normal with symptoms, FVC <50% predicted, or evidence of sleep-disordered breathing on polysomnography 5, 2

• Use bilevel positive airway pressure (BPAP) with backup respiratory rate for better patient-ventilator synchrony, particularly in patients with bulbar impairment 5
• Establish baseline pulmonary function with slow vital capacity (SVC) measurements and peak cough flow (PCF) to assess airway clearance ability 2
• NIV improves both survival and quality of life when implemented appropriately 6
• Critical caveat: Compliance with NIV is adversely affected by cognitive impairment, requiring careful assessment before implementation 7

Nutritional Support

Assess nutritional status (BMI, weight loss) every 3 months to detect early malnutrition, with interventions escalating based on severity 2, 5

Staged Nutritional Interventions:

• Early dysphagia: Adapt food texture to facilitate swallowing, implement chin-tuck postural maneuvers, and use thicker liquids and semisolid foods with high water content instead of thin liquids 2, 8, 5
• Moderate dysphagia: Fractionate and enrich meals with energy or deficient nutrients; add oral nutritional supplementation if weight loss progresses 8
• Severe dysphagia: Perform videofluoroscopy (VFS) at diagnosis to detect early dysphagia, even in asymptomatic patients with bulbar symptoms 2, 8

Gastrostomy Placement:

Place percutaneous endoscopic gastrostomy (PEG) before respiratory function significantly deteriorates, ideally when FVC remains >50% of predicted; refuse gastrostomy when FVC falls below 30% 5, 2

• Enteral nutrition via feeding tubes (preferably gastrostomy) is recommended when oral intake becomes insufficient 2, 8
• Parenteral nutrition should only be considered in acute settings when enteral nutrition is contraindicated, as home parenteral nutrition has high complication rates (3.11 per 1000 catheter days) 8

Symptomatic Management

Most Prevalent and Bothersome Symptoms:

Fatigue (90% prevalence, 18% most bothersome) is the most common yet most undertreated symptom in ALS, requiring aggressive management despite limited evidence-based interventions 9

• Muscle stiffness affects 84% of patients (14% most bothersome) 9
• Muscle cramps occur in 74% of patients 9
• Shortness of breath is bothersome in 12% of patients 9

Bulbar Symptom Management:

• First-line therapy for sialorrhea: Inexpensive oral anticholinergic medication 5
• Second-line therapy for sialorrhea: Botulinum toxin therapy to salivary glands 5
• All patients with suspected bulbar dysfunction require swallow screening before initiating oral intake 5
• Patients with positive bedside screening or high aspiration risk require videofluoroscopy swallowing study (VFSS)/modified barium swallow 5

Physical Activity:

• Advise low to moderate physical activity as long as it doesn't worsen the patient's condition, as endurance and resistance exercises may slow disease progression and improve functionality 8
• Avoid excessive physical exertion that could lead to fatigue and worsen symptoms 8

Cognitive and Behavioral Management

Screen for cognitive impairment and frontotemporal dementia, as these significantly impact survival, treatment compliance, caregiver burden, and safety 7

• ALS patients with executive dysfunction are at higher risk of falls, choking episodes, and injuries 7
• Behavioral deficits have significant negative impact on caregivers' quality of life and increase carer burden considerably 7
• Patients with cognitive impairment show poorer compliance with walking aids, PEG feeding tubes, and other safety devices 7
• Critical consideration: Insistence on NIV and feeding tube placement in the presence of significant behavioral and cognitive deficits can be inappropriate and should be carefully evaluated 7
• A specialist clinical neuropsychologist should be available as part of the multidisciplinary ALS team 7

Palliative Care Integration

Adopt a palliative care approach from the time of diagnosis, not reserved for end-stage disease, with early referral to palliative services to establish relationships before communication becomes severely limited 2, 5, 7

Timing of End-of-Life Discussions:

• Initiate end-of-life discussions at specific trigger points: presence of patient distress, evolution of disease, or expressed desire of the patient to discuss these issues 7
• Early discussions are essential because speech and communication become severely limited in later stages 5
• Cross-cultural data shows significant variation in end-of-life discussion timing (mean 5 months prior to death in UK vs. 10 months in USA) 7

Multidisciplinary Team Composition

Multidisciplinary care improves both survival and quality of life in ALS; the team must include neurology, pulmonology, gastroenterology, speech-language pathology, nutrition, physical therapy, occupational therapy, social work, and palliative care 5, 6

Critical Pitfalls to Avoid

• Equipment and service delays: All requests for equipment and services for ALS should be considered urgent and handled expeditiously, as delays can result in catastrophic safety risks rather than simple inconvenience 7
• Late palliative care referral: Access to palliative care and hospice services often occurs too late in the disease course, if at all 7
• Undertreating fatigue: Despite being the most prevalent and bothersome symptom, fatigue is treated in only 10% of patients 9
• Ignoring cognitive changes: Cognitive dysfunction must be considered when approaching difficult management decisions in later disease stages 7
• Delayed gastrostomy: PEG placement must occur before FVC drops below 50% predicted to avoid increased procedural risk 5